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IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis.

Kahn A, Yadav AD, Harrison ME - Case Rep Gastrointest Med (2015)

Bottom Line: The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria.The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation.Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Mayo Clinic, Scottsdale, AZ, USA.

ABSTRACT
IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

No MeSH data available.


Related in: MedlinePlus

MRCP findings: Case 1. (a) Narrowing and stricture of the common bile duct with dilated intrahepatic ducts. (b) Resolution of intrahepatic duct dilation and CBD narrowing after steroid therapy. (c) 2.7 cm hypointense lesion in the pancreatic head (black arrow) near a region of normal pancreatic parenchyma (white arrow). (d) Resolution after steroid therapy. The pancreatic parenchyma is homogeneous and isointense with the adjacent liver.
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fig1: MRCP findings: Case 1. (a) Narrowing and stricture of the common bile duct with dilated intrahepatic ducts. (b) Resolution of intrahepatic duct dilation and CBD narrowing after steroid therapy. (c) 2.7 cm hypointense lesion in the pancreatic head (black arrow) near a region of normal pancreatic parenchyma (white arrow). (d) Resolution after steroid therapy. The pancreatic parenchyma is homogeneous and isointense with the adjacent liver.

Mentions: Laboratory workup revealed an elevated total bilirubin 8.5 mg/dL, direct bilirubin 5.2 mg/dL, alkaline phosphatase 372 IU/L, alanine aminotransferase (ALT) 299 IU/L, aspartate aminotransferase (AST) 143 IU/L, amylase 70 μ/L, and lipase 54 μ/L. His platelet count was 258 and International Normalized Ratio (INR) 0.9. Carbohydrate antigen (CA) 19-9 was 175 U/mL and carcinoembryonic antigen (CEA) level 1.1 ng/mL. Total IgG was 1,550 mg/dL and IgG4 levels were 51.8 (reference range 2.4–121). Other serologic tests included anti-neutrophil antibody (ANA), anti-smooth muscle antibody (ASMA), and anti-mitochondrial antibody (AMA), all of which were negative. A computed tomography (CT) of the abdomen demonstrated diffuse intrahepatic biliary ductal dilatation. Magnetic resonance imaging (MRI) revealed an area of enhancement concerning for neoplasm at the biliary confluence, narrowing of the central right and left intrahepatic bile ducts and intrahepatic biliary duct dilation (Figure 1(a)). Diffuse pancreatic enhancement was noted. An ERCP was performed and demonstrated a complex narrow biliary stricture involving both right and left hepatic ducts and extending to the common hepatic duct, consistent with type 4 pattern cholangiography [5]. Biliary brushings and FISH were negative for malignancy. IgG4 immunostaining of biliary brushing specimen was not pursued. Subsequently our patient underwent three ERCPs with biliary dilation/stent exchange and biliary brushings for cytology and FISH, all of which revealed no evidence of malignancy.


IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis.

Kahn A, Yadav AD, Harrison ME - Case Rep Gastrointest Med (2015)

MRCP findings: Case 1. (a) Narrowing and stricture of the common bile duct with dilated intrahepatic ducts. (b) Resolution of intrahepatic duct dilation and CBD narrowing after steroid therapy. (c) 2.7 cm hypointense lesion in the pancreatic head (black arrow) near a region of normal pancreatic parenchyma (white arrow). (d) Resolution after steroid therapy. The pancreatic parenchyma is homogeneous and isointense with the adjacent liver.
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4561869&req=5

fig1: MRCP findings: Case 1. (a) Narrowing and stricture of the common bile duct with dilated intrahepatic ducts. (b) Resolution of intrahepatic duct dilation and CBD narrowing after steroid therapy. (c) 2.7 cm hypointense lesion in the pancreatic head (black arrow) near a region of normal pancreatic parenchyma (white arrow). (d) Resolution after steroid therapy. The pancreatic parenchyma is homogeneous and isointense with the adjacent liver.
Mentions: Laboratory workup revealed an elevated total bilirubin 8.5 mg/dL, direct bilirubin 5.2 mg/dL, alkaline phosphatase 372 IU/L, alanine aminotransferase (ALT) 299 IU/L, aspartate aminotransferase (AST) 143 IU/L, amylase 70 μ/L, and lipase 54 μ/L. His platelet count was 258 and International Normalized Ratio (INR) 0.9. Carbohydrate antigen (CA) 19-9 was 175 U/mL and carcinoembryonic antigen (CEA) level 1.1 ng/mL. Total IgG was 1,550 mg/dL and IgG4 levels were 51.8 (reference range 2.4–121). Other serologic tests included anti-neutrophil antibody (ANA), anti-smooth muscle antibody (ASMA), and anti-mitochondrial antibody (AMA), all of which were negative. A computed tomography (CT) of the abdomen demonstrated diffuse intrahepatic biliary ductal dilatation. Magnetic resonance imaging (MRI) revealed an area of enhancement concerning for neoplasm at the biliary confluence, narrowing of the central right and left intrahepatic bile ducts and intrahepatic biliary duct dilation (Figure 1(a)). Diffuse pancreatic enhancement was noted. An ERCP was performed and demonstrated a complex narrow biliary stricture involving both right and left hepatic ducts and extending to the common hepatic duct, consistent with type 4 pattern cholangiography [5]. Biliary brushings and FISH were negative for malignancy. IgG4 immunostaining of biliary brushing specimen was not pursued. Subsequently our patient underwent three ERCPs with biliary dilation/stent exchange and biliary brushings for cytology and FISH, all of which revealed no evidence of malignancy.

Bottom Line: The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria.The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation.Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Mayo Clinic, Scottsdale, AZ, USA.

ABSTRACT
IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

No MeSH data available.


Related in: MedlinePlus